STAR Protocols (Jun 2023)

Generation of human iPSC-derived neurofibromaspheres for in vitro and in vivo uses

  • Helena Mazuelas,
  • Itziar Uriarte-Arrazola,
  • Juana Fernández-Rodríguez,
  • Miriam Magallón-Lorenz,
  • Alberto Villanueva,
  • Conxi Lázaro,
  • Bernat Gel,
  • Eduard Serra,
  • Meritxell Carrió

Journal volume & issue
Vol. 4, no. 2
p. 102198

Abstract

Read online

Summary: Neurofibromas are benign peripheral nervous system tumors associated with neurofibromatosis type 1, which originate from NF1(−/−) Schwann cell precursors. We describe a protocol to generate neurofibromaspheres by differentiating NF1(−/−) Schwann cells from induced pluripotent stem cells and combining them with neurofibroma primary fibroblasts. We also describe the development of neurofibroma-like tumors when neurofibromaspheres are engrafted in the sciatic nerve of nude mice. This model constitutes a versatile platform for drug screening and the study of neurofibroma biology.For complete details on the use and execution of this protocol, please refer to Mazuelas et al. (2022).1 : Publisher’s note: Undertaking any experimental protocol requires adherence to local institutional guidelines for laboratory safety and ethics.

Keywords